You are on page 1of 7

CHAPTER 166: KAWASAKI DISEASE

 Formerly known as o Persistent fever


mucocutaneous lymph node o Poor response to IVIG
syndrome and infantile o Lab abn: WBC, plt, Na.
polyarteritis nodosa albumin,CRP,
 Highest incidence in Asian transaminitis
children
 Vasculitis with a predilection for PATHOLOGY
coronary arteries  Predominantly affects medium-
 Untreated children: develop sized arteries
coronary artery abnormalities  Coronary arteries: most
including aneurysm; If treated commonly involved, others:
with IVIG <5% will develop CAA popliteal and brachial
 3 phase process to arteriopathy
ETIOLOGY of KD:
 Unknown, but certain o 1st phase – neutrophilic
epidemiologic and clinical necrotizing arteritis
features support an infectious occurring in the 1st 2 weeks
origin of illness that begins in
endothelium and moves
 Features: through the coronary wall
o Young age group - Saccular
o Wave-like geographic aneurysms may
spread of illness form
o Self-limited nature of o 2nd phase –
acute febrile illness subacute/chronic
o Clinical features of rash, vasculitis driven by
fever, enanthem, lymphocytes, plasma cells,
conjunctival injection, and eosinophils which may
cervical last to weeks or years
lymphadenopathy - Fusiform
o Infrequent if <3 mo d/t aneurysms
maternal Ab o 3rd phase – smooth muscle
o No single infectious cell myofibroblast will
etiologic agent has been develop which can cause
identified progressive stenosis
o Genetics: ITPKC gene- - Thrombi may
increased susceptibility form in the
and more severe disease lumen and
obstruct blood
EPIDEMIOLOGY flow
 M > F; mean age 3 yo
 Kobayashi score: high sensitivity MANIFESTATIONS
and specificity on Japanese  high fever (>38.3C), unremitting
population only and unresponsive to antibiotics
 Predictors of poor outcome: lasting atleast 5 days. If no
o Young age treatment, fever may last up to 4
o Male weeks

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

 In addition to fever , 5 criteria axillary, iliac or other


o Bilateral non-exudative arteries
conjunctival injection with
limbal sparing o Cardiac involvement –
o Erythema of oral and most important
pharyngeal mucosa with manifestation
strawberry tongue and red  Myocarditis occurs
cracked lips in most and
o Non-suppurative cervical manifest as
lymphadenopathy, usually tachycardia
unilateral with node size disproportionate to
>1.5 cm fever, along with
o Edema and erythema of diminished left
the hands and feet ventricular systolic
o Rash of various forms function
(maculopapular,  KD shock syndrome
erythema multiforme or – KD patients with
scarlantiniform) cardiogenic shock
 Pericarditis with
 Symptoms other than criteria: small pericardial
o GI symptoms: vomiting, effusion
diarrhea or abd pain  Mitral regurgitation
o Respi symptoms:  Giant coronary
rhinorrhea or cough aneurysms (>8mm
o Irritability which may be a internal diameter) –
consequence of aseptic greatest risk for
meningitis, mild hepatitis, rupture, stenosis,
hydrops of the and MI
gallbladder, urethritis, and
meatitis with sterile pyuria Node-first KD – 2-4 yo with fever and
and arthritis lymphadenopathy presentation only
o Localized pulsating mass –
dilatation of popliteal,

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

3 phase process:
Acute febrile phase Subacute phase Convalescent phase
Fever and other acute Desquamation, All clinical signs have
signs that lasts up to 2 wks thrombocytosis and disappeared and
CAA develop in up to 25% continues until ESR returns
perineal desquamation of untreated patients to normal 6-8 wks after
onset
arthritis and periungual
desquamation of fingers
and toes which may
progress the entire hand
and foot

Laboratory and Radiology Findings


 2D echo - most useful test to monitor CAA. Aneurysms may be small (<5mm
internal diameter), or giant (>8 mm internal diameter)
- Should be done at diagnosis and again after 2-3 wks of illness. If
normal, a repeat should be done 6-8 wks after
- If abn 2D echo or with recurrent fever – more frequent 2d echo
- 1 yr later – 2d echo and lipid profile then cardio follow up every
5 yrs
 WBC with predominance of neutrophils and immature forms
 Normochromic anemia
 Plt – normal in 1st wk then  in 2-3 wks
 ESR and CRP
 Sterile pyuria, mild elevations of hepatic transaminases, hyperbilirubinemia, CSF
pleocytosis

DIAGNOSIS
 Based on presence of clinical signs
Classic KD Atypical or Incomplete
Presence of fever for atleast 4 days Fever but fewer than 4 of the 5
and atleast 4 of 5 of other principal
characteristics Infants – highest likelihood of CAA

Do labs and 2d echo to assist in dx

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD


CHAPTER 166: KAWASAKI DISEASE

TREATMENT  Persistent or recrudescent


A.IVIG fever 36h after completion
 2g/kg of IVIG and high dose of the intial IVIG infusion
aspirin 80-100 mg/kg/day Q6  Have increased risk for
within 10 days of disease onset CAA
 IVIG results in defervescence and  Give another dose of IVIG
resolution of clinical signs of illness 2g/kg
in 85% of patients
 Defer MMRV vaccine until 11 mos COMPLICATIONS
after IVIG administration. Non-live  Small solitary aneurysm –
vaccines do not need to be continue Aspirin indefinitely
deferred  Larger or numerous aneurysm –
require addition of other
B.Aspirin antiplatelet agents or
 decreased from anti- anticoagulation
inflammatory dose to anti-  Long term: periodic echo with
thrombotic dose (3-5 mg/kg/day stress testing and possible
as a single dose) after the patient angiography if large aneurysm
has been afebrile for 48 hrs, are present
although some prescribe until 14th  Coronary stenosis: catheter
day of illness intervention with percutaneous
 continued for 6-8 wks after illness transluminal coronary rotational
onset and dc in patients who ablation, directional coronary
have had normal 2d echo atherectomy, and stent
 If with CAA, Aspirin is continued implantation
and may require
anticoagulation, depending on PROGNOSIS
the degree of coronary dilation  Depends on the severity of
 If long term: give annual Influenza coronary disease
vaccination to reduce Reye  Acute KD recurs in 1-3% of cases
syndrome  50% of coronary aneurysms
regress to normal lumen diameter
C.Corticosteroids by 1-2 yrs after the illness, with
Single pulse dose of IV smaller aneurysms more likely to
methylprednisone 2mg/kg plus IVIG regress
 Giant aneurysms are less likely to
IVIG-resistant KD regress and may lead to
 Occurs in 15% of patients thrombosis and stenosis. CABG(
arterial grafts) may be required if
myocardial perfusion is impaired

CHAPTER 166: KAWASAKI DISEASE | By: Hazel Anne M. Endaya, MD